Simultaneous thrombosis of cerebral artery and venous sinus

Cerebral venous thrombosis (CVT) is infrequent among cerebrovascular diseases. The simultaneous thrombosis involving both cerebral artery and venous sinus is even extremely rare. We reported a 41-year-old woman who presented with acute headache and left hemiparesis due to concomitant arterial ischemic stroke and recurrent CVT. Extensive investigation disclosed acquired protein C and protein S deficiency, iron deficiency anemia (IDA) and cryoglobulinemia. She was treated with intravenous injection of heparin followed by oral anticoagulant therapy. The headache rapidly subsided; however, left hemiparesis persisted over five months. The rare condition of simultaneous thrombosis of cerebral artery and venous sinus may be caused by the synergistic effect of coagulation disorders, IDA and cryoglobulinemia.     

Isolated Lateral Sinus Thrombosis Presenting as Cerebellar Infarction in a Patient with Iron Deficiency Anemia

As a rare cerebrovascular disease, cerebral venous thrombosis (CVT) is caused by various conditions including trauma, infection, oral contraceptive, cancer and hematologic disorders. However, iron deficiency anemia is not a common cause for CVT in adult. Posterior fossa infarction following CVT is not well demonstrated because posterior fossa has abundant collateral vessels. Here, we report a case of a 55-year-old man who was admitted with complaints of headache, nausea, and mild dizziness. The patient was diagnosed with isolated lateral sinus thrombosis presenting as cerebellar infarction. Laboratory findings revealed normocytic normochromic anemia due to iron deficiency, and the patient''s symptoms were improved after iron supplementation.     

A Case of Cerebral Venous Thrombosis and Deep Venous Thrombosis Due to Hyperthyroidism with Increased Factor VIII Activity

A 48-year-old woman was admitted to our hospital because of headache and fever. She was diagnosed with aseptic meningitis. Five days later, she had a seizure and developed left hemiparesis. Magnetic resonance imaging showed hyperintensity in the right parietal area on fluid attenuated inversion recovery imaging. She was diagnosed as having cerebral venous thrombosis (CVT) because the suprasagittal sinus was invisible on the venographic studies. Moreover, deep venous thrombosis (DVT) was detected in her left lower extremity. Laboratory findings showed hyperthyroidism and markedly increased factor VIII activity. This is a rare case of concomitant CVT and DVT induced by high factor VIII activity due to hyperthyroidism under the presence of meningitis, an additional risk factor for thrombosis.     

Neuropsychological manifestations in a case of bilateral thalamic infarction.

Neuropsychological manifestation has been reported with lesions of the anterior and non-specific thalamic nuclei and mammilothalamic tract (MMT). These have been reported in the setting of arterial infarction and/or haemorrhage. Cerebral venous sinus thrombosis (CVT) is a rare cause of brain infarction. It occurs in the setting of oral contraceptive administration or pregnancy. Inherited thrombophilias are documented risk factors. The most frequent being heterozygous factor V Leiden mutation. We report a single case of bilateral thalamic infarction due to cerebral vein and sinus thrombosis. Clinically the case manifested with memory impairment and dysexecutive symptoms. Predisposing factor for venous thrombosis was a homozygous factor V Leiden mutation. The patient was treated with anticoagulation and made a good recovery.     

Vernet's Syndrome Associated with Internal Jugular Vein Thrombosis

Our objective is to present a case of Vernet's syndrome (cranial nerve (CN) IX, X, and XI palsy) associated with cerebral venous thrombosis (CVT) in an internal jugular vein. The patient presented with acutely developed dysphagia. The weakness of the left sternocleidomastoid and trapezius muscles was observed. The initial magnetic resonance imaging and computed tomography (CT) with contrast enhancement showed contrast-filling defect in the left internal jugular vein inside the jugular foramen. The magnetic resonance venography with contrast enhancement revealed a partial filling defect in the left sigmoid sinus and total occlusion of the left internal jugular vein. Under the diagnosis of CVT associated with CN IX, X palsy, anticoagulation therapy with low-molecular-weighted heparin was initiated. Despite the continued anticoagulation therapy for 3 months, neither the burden of thrombosis in the left sigmoid sinus and internal jugular vein on neck CT nor dysphagia symptoms improved. Clinicians need to be aware of internal jugular venous thrombosis as one of the differential diagnoses in Vernet's syndrome in patients in a hypercoagulable state. Further reporting of similar cases is needed to confirm the association between CVT and Vernet's syndrome.     

Cerebral venous thrombosis: a young woman case study

Cerebral venous sinus thrombosis is a rare, serious cerebrovascular disease with poor prognosis. It can be a sequel to various coagulation disturbances, head injuries or local inflammations. We report a case of a young woman with no risk factors detected, who developed a massive cerebral venous sinus thrombosis. She had progressively worsening symptoms, including left hemiplegia, aphasia, tonic-clonic seizures and unconsciousness. The diagnosis was supported by CT, MRI and angio-MRI findings. The intensive i.v. heparin and streptokinase treatment, as well as antibiotics, resulted in full remission of all patient's symptoms. The case emphasizes the necessity of early diagnosis and management of cerebral venous sinus thrombosis.     

Interventions in cerebrovascular emergencies among patients with Paroxysmal nocturnal haemoglobinuria - A word of caution

IntroductionParoxysmal nocturnal haemoglobinuria (PNH) is a clonal hematopoietic disorder, where there is deficiency of glycosylphosphatidylinositol (GPI) anchored proteins in the cell membrane, leading to increased complement sensitivity of red blood cells, intravascular hemolysis and vascular inflammation. Arterial and venous strokes in patients with PNH are a rarity posing significant diagnostic and therapeutic challenges. We report our experience with management of PNH patients with cerebrovascular emergencies.MethodsWe report 2 patients with PNH, one who was previously diagnosed with PNH and had arterial stroke, the other had an index presentation of cerebral venous sinus thrombosis (CVT) and was subsequently diagnosed with PNH. We also present the systematic review of literature reporting similar cases, highlighting the challenges in management.ResultsBoth patients presented to our centre with cerebrovascular emergency. The first patient was a diagnosed with PNH, and presented with left hemispheric infarction caused by thrombosis of middle cerebral artery. He was thrombolysed and underwent mechanical thrombectomy, which was unsuccessful in view of repeated re – thrombosis of the vessel. The patient survived with significant disability. The second patient had severe cerebral venous sinus thrombosis with large right hemispheric hemorrhagic venous infarction. She underwent emergency decompressive hemicraniectomy complicated by massive blood loss and disseminated intravascular coagulation. She subsequently had recurrent life threatening intracranial bleed secondary to platelet transfusions, thrombocytopenia, and use of contrast agents. She progressed to develop Budd Chiari syndrome and was initiated on Eculuzimab. She became transfusion independent, however remained in minimally conscious state and succumbed to sepsis.ConclusionsManagement of arterial and venous strokes is complex in patients with PNH. Invasive procedures and platelet transfusions are to be avoided in acute thrombosis, till robust evidence is available establishing the safety of the same in patients with PNH. Eculuzimab is a promising option, but far from reach for patients in developing countries.     

脑静脉窦血栓形成的诊断与血管内治疗

目的 探讨脑静脉窦血栓形成的诊断和血管内溶栓治疗。方法 对8例以顽固性头痛、呕吐伴或不伴局灶性神经功能缺损或癫发作为主要表现的患者，采用颅脑MRI和脑血管造影检查确定诊断，并施行经颈内动脉、静脉窦内接触性溶栓或静脉窦内支架成形术协同抗凝治疗。结果 MRI显示相应脑静脉窦区出现长T2WI，短T1WI信号，DSA显示脑静脉窦闭塞、静脉侧支循环建立和脑动静脉循环时间延长。治疗后静脉窦完全或部分再通，脑循环时间接近正常，临床病情显著缓解。结论 血管内介入治疗可能是脑静脉窦血栓形成最有效的治疗方法之一，有待大样本研究证实。     

Cerebral venous sinus thrombosis and thrombophilia presenting as pseudo-tumour syndrome following mild head injury

Cerebral venous sinus thrombosis (CVT) after mild head injury is infrequent. A 38-year-old patient presented with a Glasgow Coma score (GCS) of 15 after a road traffic accident. CT scan revealed a temporal contusion. He was treated with measures to prevent cerebral oedema and anticonvulsants. Three weeks later he presented with features of pseudo-tumour syndrome. Investigations revealed the presence of cerebral venous sinus thrombosis, protein C deficiency and elevated titres of antiphospholipid antibodies. He was treated with anticoagulants and showed improvement. This case report highlights that multiple "hits" may lead to CVT and hence laboratory screening of patients with CVT is necessary even if the clinical situation seemingly provides sufficient explanation for a thrombotic event. The presence of acquired and inherited causes of thrombophilia need not always lead to symptomatic thrombosis. As illustrated by this case, a second hit, such as trauma, may be the precipitating factor that unmasks the prothrombotic state.     

Deep cerebral vein thrombosis associated with iron deficiency anaemia in adults.

Cerebral venous thrombosis (CVT) is rare and has a wide spectrum of symptoms, therefore it is difficult to diagnose. Thrombosis of the deep cerebral veins occurs very rarely: it has been reported that approximately 6% of patients with CVT have deep CVT, and the prognosis for patients with this condition is poor. CVT has been reported in association with dehydration, a hypercoagulable state, mastoiditis, tumour invasion of a venous sinus, use of oral contraceptives, pregnancy, puerperium, head trauma, vasculitis, and intracranial and systemic infections. However, in the literature, there are few reported cases of CVT in association with iron deficiency anaemia, especially in adults. We present here two patients with bilateral thalamic and basal ganglionic lesions due to thrombosis of the deep cerebral veins. Both of our patients had severe hypochromic microcytic anaemia due to iron deficiency, and both had a good prognosis after 2 months.     

Facial palsy in cerebral venous thrombosis : transcranial stimulation and pathophysiological considerations

BACKGROUND: Cranial nerve palsy in cerebral sinovenous thrombosis (CVT) is rare, its pathophysiology remains unclear, and data from electrophysiological examinations in such patients are missing. CASE DESCRIPTION: We report the case of a 17-year-old woman with familial protein S deficiency who was admitted with extensive multiple CVT. Two weeks after onset of symptoms, she developed isolated right peripheral facial palsy, and MR venography showed segmental occlusion of the ipsilateral transverse sinus. Complete recovery of facial palsy occurred concomitant with recanalization of the transverse sinus. Facial neurography, including transcranial magnetic stimulation of the facial nerve and related motor cortex, ruled out a coincidental idiopathic palsy and revealed conduction block proximal to the facial canal. CONCLUSIONS: Facial palsy in our patient was caused by transient neurapraxia in the intracranial segment of the nerve. We suggest that elevated venous transmural pressure in the nerve's satellite vein, which belongs to the affected drainage territory of the transverse sinus, might have caused venous blood-brain barrier dysfunction in the intrinsic vascular system of the nerve, with leakage of fluids and ions into the endoneurial space and thus an increase in interstitial resistance.     

Cerebral venous thrombosis secondary to essential thrombocythemia

INTRODUCTION: Essential thrombocythemia (ET) is a myeloproliferative syndrome; cerebral venous thrombosis (CVT) is a rare complication. OBSERVATION: We report the case of a 20-year-old woman with an uneventful history who was admitted with intracranial hypertension syndrome which had developed over the last four months in association with bilateral decline of visual acuity. Physical examination at admission revealed stage II papilloedema and absence of any focal neurological signs. The brain MRI and the venous MRA showed a thrombosis involving the superior longitudinal sinus and the lateral sinus. The etiological analysis disclosed essential thrombocythemia (ET). The patient was given an antiedema and anticoagulant treatment. Later, an etiological cytoreductive therapy was initiated. Signs of intracranial hypertension regressed progressively with persistence of acute visual disorders associated with sequelar optical atrophy. Discussion. ET is a rare cause of CVT. Reports in the literature have discussed the mechanisms, the physiology, the therapeutic modalities and the clinical course of these CVTs secondarily to ET. CONCLUSION: Stroke, especially transient ischemic attack, is the usual thrombotic expression of myeloproliferative syndrome. CVT is much more exceptional.     

Cerebral Venous Thrombosis after Red Blood Cell Transfusion for the Treatment of Iron Deficiency Anemia

A 57-year-old male presented with generalized seizure who received red blood cell (RBC) transfusion for the treatment of iron deficiency anemia (IDA). Neuroradiological findings revealed cerebral venous thrombosis (CVT) on the left frontal vein. He received anticoagulants, anticonvulsants, and iron supplements. He discharged without any neurological deficit. It should be noted that RBC transfusion might increase the risk of CVT in patients with IDA.     

An unusual cause of cerebral venous sinus thrombosis. Paroxysmal nocturnal hemoglobinuria

Cerebral venous sinus thrombosis caused by paroxysmal nocturnal hemoglobinuria is uncommon. Our case is a 44-year-old woman who presented with a 2 day history of headaches, nausea, and seizures followed by a Todd`s paresis; she had been diagnosed as paroxysmal nocturnal hemoglobinuria for 4 years. A magnetic resonance venography revealed extensive thrombosis of the cerebral venous sinus. She received antithrombotic treatment with a good outcome. We highlight paroxysmal nocturnal hemoglobinuria as the reason for the cerebral venous sinus thrombosis. The treatment of cerebral venous sinus thrombosis caused by paroxysmal nocturnal hemoglobinuria should be individualized.     

Homocystinuria with transverse sinus thrombosis

A case of cerebral venous thrombosis caused by undiagnosed homocystinuria is reported. The pitfalls regarding the diagnosis of a potentially medically treatable condition are discussed. Cerebral venous thrombosis in children has a variable type of onset and a multiplicity of causes. This type of pathology, although not frequent, is more common than previously thought. Among the different etiologies, undiagnosed homocystinuria is not routinely considered. We report a case of venous thrombosis of the left transverse cerebral sinus in a girl with drug-resistant partial epilepsy and homocystinuria. This diagnosis was considered and confirmed after the appearance of acute cerebral symptoms caused by venous thrombosis.     

Interobserver agreement in the magnetic resonance location of cerebral vein and dural sinus thrombosis

The interobserver variation in the magnetic resonance (MR) location of cerebral vein and dural sinus thrombosis (CVT) has not been previously reported. Four independent observers rated a convenience sample of 40 MR/MR angiographies to assess whether or not each dural sinus and major cerebral veins were occluded. Interobserver reliability was measured using κ statistics. Interobserver agreement was comparable between the six pairs of raters. Agreement was excellent for thrombosis of the deep cerebral venous system ( κ  = 1.00), cerebellar veins ( κ  = 1.00), superior saggital sinus ( κ range: 0.82–1) and right jugular vein ( κ range: 0.84–0.95); good to excellent for the right transverse/sigmoid sinus ( κ range: 0.75–0.90) and the left jugular vein ( κ range: 0.65–0.85); moderate to excellent for the left lateral sinus ( κ range: 0.59–0.78) and the straight sinus ( κ range: 0.59–0.92); poor to good for the cortical veins ( κ range: 0.02–0.65). Agreement between observers varies with the location of CVT. It is good or excellent for most of the occluded sinus and veins, except for the cortical veins. This study suggests that information on the location of CVT can be reliably collected and used in multicentre studies.     

Cerebral sinovenous thrombosis in children: another reason to treat iron deficiency anemia

Iron deficiency anemia is a rare cause of cerebral sinovenous thrombosis in children. We report three cases of cerebral sinovenous thrombosis and iron deficiency anemia treated at Primary Children's Medical Center in Salt Lake City, Utah, between 1998 and 2001. The children were 9, 19, and 27 months old at the time of admission. Hemoglobin levels ranged from 6.6 to 7.0 g/dL, mean corpuscular volume levels from 45 to 56 fL, and platelet counts from 248,000 to 586,000/microL. Magnetic resonance imaging and magnetic resonance venography revealed thrombosis of the straight sinus and internal cerebral veins in all three children, with the addition of the vein of Galen, left transverse and sigmoid sinuses, and upper left internal jugular vein in one child. Recovery ranged from excellent to poor in 3 months to 3 years of follow-up. Four additional cases, ages 6 to 22 months, were found in the English-language literature. Evaluation for prothrombotic disorders was negative in all children, including the current cases. Treatments have included thrombectomy, corticosteroids, mannitol, heparin, low-molecular-weight heparin, warfarin, aspirin, blood transfusion, and iron supplementation, but there is no consensus regarding therapy, other than to correct the anemia and treat iron deficiency. Iron deficiency anemia, a preventable cause of cerebral sinovenous thrombosis, deserves consideration when cerebral sinovenous thrombosis is detected in young children.     

以头痛为唯一临床表现的脑静脉窦血栓形成患者的头痛特征

目的 探讨以头痛为唯一临床表现的脑静脉窦血栓形成（cerebral venous thrombosis,CVT）患者的头 痛特征。 方法 回顾性纳入2012年1月-2018年12月北京航天总医院神经内科连续收治的以头痛为唯一临床 表现的CVT患者。以头痛为唯一表现指除头痛外无提示颅内压升高的体征,且脑脊液压力正常,影像 或脑脊液检测显示无蛛网膜下腔出血。分析这类患者的头痛特征（部位、发作模式、演变和强度）。 结果 共8例患者纳入本研究,中位年龄40岁,女性6例（75%）。最常受累的静脉窦是侧窦（7例, 87.5%）。4例患者累及左侧侧窦,头痛均位于左侧;4例患者累及中线静脉窦（上矢状窦或直窦）,头痛 均为双侧性。头痛发作模式：进展性头痛7例,闪电样头痛1例;演变：持续性7例,间歇性1例;强度：严 重头痛7例,中等程度头痛1例。 结论 对于仅表现为头痛并且颅内压正常以及无蛛网膜下腔出血的CVT患者,侧窦受累很常见,尤 其是左侧。头痛部位与受累静脉窦位置一致。     

A case of venous dural sinus thrombosis presenting dementia syndrome. An autopsy case

We report clinical and pathological features of a case of dementia syndrome due to dural sinus thrombosis. In three years before admission, the patient, a 64-year old man, had had four convulsive attacks; scattered calcification shadows were disclosed by plain CT. Ten months before the admission, he complained of progressive memory disturbance and dyscalculia. General physical examination showed no remarkable abnormalities except for bruit at the left mastoid process. Although his time orientation was poor, he was otherwise fully awake. Neurologically, memory disturbance, finger agnosia, and dyscalculia were observed. Right Barré's sign, and exaggerated right patellar tendon reflex were observed. Plain X-ray CT revealed calcification-like, scattered high-density areas in the floor of the cerebral cortical sulci. Enhanced CT showed abnormal vessel high-density areas in both the cerebrum and brainstem. Cerebral angiography showed thrombosis of bilateral transverse sinuses, arterio-venous fistula in the left transverse sinus, and remarkably dilated cortical veins over both cerebral hemispheres. Positron emission tomography revealed misery perfusion areas in bilateral cerebral hemispheres. We presume that the mass effect of venous engorgement and chronic ischemia due to the arterio-venous fistula caused the dementia syndrome. These clinical manifestations were improved by amantadine and the patient was discharged. During follow-up, he died of subarachnoid hemorrhage, and an autopsy was performed. Pathological findings were as follows: 1) cortical and subcortical multiple-infarction in the cerebrum, 2) hematoma in the subarachnoid space, 3) venous dilatation of the cortical veins and pseudocalcification of their walls, and 4) thrombus in the transverse dural sinus.     

Locked-in syndrome due to tentorial herniation

A 28-year-old man had a chronic locked-in syndrome following tentorial herniation caused by an epidural hematoma. Postmortem examination revealed bilateral corticospinal tract degeneration caudal to the midbrain, with infarction of the right internal capsule just rostral to the cerebral peduncle and pressure necrosis of the pyramidal portion of the left cerebral peduncle.